2. INTRODUCTION
INTRODUCTION
Paget’s disease is a chronic condition of bone
characterized by disorder of the normal bone
remodeling process.
Characterized by excessive breakdown of bone
tissue followed by abnormal bone formation.
Also known as Osteitis Deformans is a bone
disease unknown cause.
Affecting men twice as freaquently as women.
Named after the England Surgeons Sir James
Paget.
3. DISEASE CLASSIFICATION
DISEASE CLASSIFICATION
INFLAMMATORY
DISEASE
•Multifocal chronic
skeletal disease due to
Chronic paramyxoviral
Virus infection.
•As a result, the bone that
is formed is abnormal.
CONGENTIAL
•Certain of genes may
Been associated with
Paget’s disease, including
The Sequestrosome 1
Gene On Chromosome 5.
4.
ETIOLOGY
ETIOLOGY
The cause of Paget’s disease is unknown.
Disease may be caused by a virus.
Paramyxovirus infection of osteoblasts may
activate of the c-fos proto-oncogene,
resulting in localized abnormal osteoclastic
activity typical of paget’s disease.
Also, a person’s genes may predispose them
to developing paget’s disease.
5. Paget’s disease affects older skeletal bone
of adults.
There is also an extremely rare form of
paget’s disease in children, referred to as
Juvenile Paget’s disease.
6. Pathogenesis
Pathogenesis
Hyper vascular/
Osteolytic phase
Initial phase of
Disorder involves
Bone resorption by
osteoclasts
Intermediate
phase
Osteoclytic activity
+
Osteoblastic activity
Exhaustive
(burn out)
stage
Abnormal matrix
Persists but cellular
Activity is nearly
Absent.
Paget’s disease
7. MANIFESTATIONS - SYMPTOMS
MANIFESTATIONS - SYMPTOMS
Paget’s disease is more commonly asymptomatic,
but may exhibit a variety of non-specific
symptoms due to increased bone turnover.
The bones that are move commonly affected
include the pelvis, spine, skull, and the long bone.
Individuals may experience any or a combination
of the following symptoms;
Bone pain
fractures
8. Skeletal Deformities – bowed legs, Fronto-
occipital skull enlargement.
Warm sensation – due to increased blood
supply to abnormal bones.
Nerve damage – hearing loss, visual
abnormalities.
Cardiac abnormalities – shortness of breath.
9. MANIFESTATIONS - SIGN
MANIFESTATIONS - SIGN
Signs may be bitemporal skull
enlargement with frontal “bossing”,
diluted scalp veins, nerve deafness in one
or both ears.
Also angioid streaks in the fundus of the
eyes and a short kyphotic trunk.
10. SIGNIFICANT LAB TESTS
SIGNIFICANT LAB TESTS
Blood chemistry (blood patient who have
paget’s disease) results indicate very high
alkaline phosphates levels with normal
serum calcium and phosphorus.
There is no known cure for this disease.
Most cases are mild and asymptomatic no
treatment is necessary in symptomatic
cases medications.
11. IMAGING CONSIDERATION
IMAGING CONSIDERATION
X-ray of the skull, spine, pelvis and long
bone.
CT scan
MRI (Magnetic Resonance Imaging)
Radionuclide bone scan or RNI
(Radionuclide Imaging).
12. RADIOLOGICAL APPEARANCE
RADIOLOGICAL APPEARANCE
Radionuclide bone scans readily detect
Paget’s disease even in its very early stage.
Radiographically the affected bone
typically demonstrate cortical thickening,
with a coarse, thickened trabecular pattern.
Mixed areas of radiolucent Osteolysis and
radiopaque Osteosclerosis may be seen.
13. Radiograph of the
fibula and tibia of the
patient in figure
demonstrating the
effect of advanced
proliferative Paget’s
disease on the fibula
and tibia.
14. Paget’s disease on pelvis…
Paget’s disease on pelvis…
Paget’s disease
involving the left
hemi pelvis and right,
there is severe
osteoarthritis of the
left hip but a
relatively normal
joint space in the
right hip.
